Hello and welcome to the Zero to Finals podcast.

My name is Tom and in this episode I'm going to be talking to you about hereditary spherocytosis.

And if you want to follow along with written notes on this topic, you can follow along at

0.0.3.3-sferocytosis

or in the hematology section of the zero-definals pediatrics book.

So let's get straight into it.

Hereditary spherocytosis is a condition where the red blood cells are sphere-shaped, making them fragile

and easily destroyed when they pass through the spleen.

It's the most common cause of inherited hemolytic anemia in northern Europeans.

It is an autosomal dominant genetic condition.

Let's talk about the presentation.

Ereditary sphero cytosis presents with jaundice, anemia, gallstones and spleenomegaly.

Patients can have episodes of hemolytic crisis, often triggered by infections,

whether hemolysis, anemia and jaundice are more significant.

Patients with hereditary sphero cytosis can develop aplastic crisis.

During aplastic crisis, there's increased anemia, hemolysis and jaundice,

without the normal response from the bone marrow of creating new red blood cells.

Usually the bone marrow will respond to hemolysis by producing

extra reticulocytes, which are immature red blood cells, which demonstrates that the bone marrow

is trying extra hard to produce extra red blood cells. In a plastic crisis, there is no reticulocyte response. This is often triggered by infection

with parvovirus. Now for a tom tip, infection with parvovirus causing a plastic crisis is a classic

exam feature of hereditary spheroocytosis. It's worth remembering this

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